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MICROBIOLOGY PAGE 1
IMMUNODEFICIENCY DISEASES
DR.TARUNIKAA G
STORY TIME
The story of David Vetter
MICROBIOLOGY
CLASS
Dr.TARUNIKAA G PAGE 2
What is
immunodeficiency
Impaired host
defence
MICROBIOLOGY
CLASS
Dr.Tarunikaa G PAGE 3
CLASSIFICATION
• PRIMARY
• SECONDARY
MICROBIOLOGY
CLASS
Dr.Tarunikaa G PAGE 4
Causes of immunodefeciency
• Humoral immunity
• Cell mediated immunity
• Combined
Specific Non-specific
• Complements
• Phagocytosis
MICROBIOLOGY
CLASS
Dr.Tarunikaa G PAGE 5
PRIMARY
IMMUNO-
DEFICIENCY
X linked Agammaglobulinaemia
Transient hypoglobulinaemia of infancy
Common variable immunodeficiency
Selective immunoglobulin deficiency
Immunodeficiencies with hyper IgM
Transcobalamin II deficiency
MICROBIOLOGY CLASS Dr.Tarunikaa G PAGE 6
X linked Agammaglobulinaemia
Burton disease
BOYS (X linked)
Bcell maturation defect
BTK gene
Depletion of cells-Bursa
dependant areas
B
6 months
virus
MICROBIOLOGY
CLASS
Dr.Tarunikaa G PAGE 7
Late onset
hypogammaglobulinaemia
• 15-35years
• Pyogenicinfections
• Autoimmunedisorders
• IncreasedsuppresorT cells
• DecreasedhelperT cells
Transient
hypoglobulinaemia
of infancy
C o m m o n v a r i a b l e
i m m u n o d e f i c i e n c y
Selective
immunoglobulin
deficiency
• Delay in synthesis
• Spontaneous recovery 18
and 30 mo.
• Rec. Otitis media
• Respiratory infections
Isolated IgA- Most common
- Respiratory infection
Selective IgM- Septicemia
MICROBIOLOGY
CLASS
Dr.Tarunikaa G PAGE 8
I m m u n o d e f i c i e n c i e s
w i t h h y p e r I g M
T r a n s c o b a l a m i n
I I d e f i c i e n c y
X linked / Ar Ar
Vitamin B12 deficiency
MICROBIOLOGY
CLASS
Dr.Tarunikaa G PAGE 9
Thymic
hypoplasia
Chronic
mucocutaneous
candidiasis
Purine nucleoside
phosphorylase
deficiency
T cell defect
• Associated
endocrinopathies
• Delayed hypersensitivity
• Intracellular killing
• Transfer factor therapy
with amphotericin B
C – Cardiac
abnormalities
A - Abnormal face
T – T cell defect.
Thymus abnormality
C – Cleft palate
H – Hypocalcemia
22 – Chr 22
Purines
Hydroxanthine
Uric acid
MICROBIOLOGY
CLASS
Dr.Tarunikaa G PAGE 10
Combined immunodeficiency
Wiskott Aldrich
syndrome
• Defective CI
• Abnormal
immunoglobulin
• Bone marrow transplant
• transfer factor
• Thymus transplantation
AR
• Cerebellar ataxia
• Telengectasia
• Ovarian dysgenesis
• Chr abnormality
Ataxia telengectasia
Nezelof's disease
AR
ECZEMA
T
P
INFECTION
Reticular dysgenesis of De Vaal
MICROBIOLOGY
CLASS
Dr.Tarunikaa G PAGE 11
Precursors of immunocompetent cells
in fetal liver and bone marrow
Multipotent hematopoietic stem cell
SCID
Swiss type agammaglobulinemial
Lymphoid stem cell
SLE
C3- Recurrent pyogenic
infection
C6 C7 C8- Neisserial infection.
01
Component
deficiency
Disorders of complement
C1 inhibitor -
Angioneurotic edema
C3b inactivator -
Pyogenic infeection.
02
Inhibitor
deficiency
• Enzyme deficiency
• Deficiency of opsonic antibody
• Drugs
• Antineutrohil autoantibodies
Disorders of
phagocytosis
Shwachman disease
Actin binding protein
deficiency syndrome
Hyper IgE syndrome
Lazy leucocyte syndrome
Tuftsin deficiency
Job syndrome
Myeloperoxidase deficiency
Leucocyte G6PD deficiency
CHronic granulomatous
infections
• Catalase positive pyogenic
pathogens - staphylococcus
and coliforms
• Diminished H2O2
• Defective release of
myeloperoxidase
• NBT test
CHEDIAK HIGASHI
SYNDROME
• Decreased pigmentation
• Photophobia
• Nystagmus
• Peroxidase positive
inclusion bodies
MICROBIOLOGY
CLASS
Dr.Tarunikaa G PAGE 16
Secondary immunodeficiency
Can live vaccines be given in
immune deficiency
patients?
Enzyme that is deficient in SCID?
What are the 5 B's in X linked
agammaglobulinemia?
MICROBIOLOGY
CLASS
Dr.Tarunikaa G PAGE
17
for listening !

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immunodeficiency diseases

  • 1. MICROBIOLOGY PAGE 1 IMMUNODEFICIENCY DISEASES DR.TARUNIKAA G
  • 3. The story of David Vetter
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  • 5. MICROBIOLOGY CLASS Dr.TARUNIKAA G PAGE 2 What is immunodeficiency Impaired host defence
  • 6. MICROBIOLOGY CLASS Dr.Tarunikaa G PAGE 3 CLASSIFICATION • PRIMARY • SECONDARY
  • 7. MICROBIOLOGY CLASS Dr.Tarunikaa G PAGE 4 Causes of immunodefeciency • Humoral immunity • Cell mediated immunity • Combined Specific Non-specific • Complements • Phagocytosis
  • 8. MICROBIOLOGY CLASS Dr.Tarunikaa G PAGE 5 PRIMARY IMMUNO- DEFICIENCY X linked Agammaglobulinaemia Transient hypoglobulinaemia of infancy Common variable immunodeficiency Selective immunoglobulin deficiency Immunodeficiencies with hyper IgM Transcobalamin II deficiency
  • 9. MICROBIOLOGY CLASS Dr.Tarunikaa G PAGE 6 X linked Agammaglobulinaemia Burton disease BOYS (X linked) Bcell maturation defect BTK gene Depletion of cells-Bursa dependant areas B 6 months virus
  • 10. MICROBIOLOGY CLASS Dr.Tarunikaa G PAGE 7 Late onset hypogammaglobulinaemia • 15-35years • Pyogenicinfections • Autoimmunedisorders • IncreasedsuppresorT cells • DecreasedhelperT cells Transient hypoglobulinaemia of infancy C o m m o n v a r i a b l e i m m u n o d e f i c i e n c y Selective immunoglobulin deficiency • Delay in synthesis • Spontaneous recovery 18 and 30 mo. • Rec. Otitis media • Respiratory infections Isolated IgA- Most common - Respiratory infection Selective IgM- Septicemia
  • 11. MICROBIOLOGY CLASS Dr.Tarunikaa G PAGE 8 I m m u n o d e f i c i e n c i e s w i t h h y p e r I g M T r a n s c o b a l a m i n I I d e f i c i e n c y X linked / Ar Ar Vitamin B12 deficiency
  • 12. MICROBIOLOGY CLASS Dr.Tarunikaa G PAGE 9 Thymic hypoplasia Chronic mucocutaneous candidiasis Purine nucleoside phosphorylase deficiency T cell defect • Associated endocrinopathies • Delayed hypersensitivity • Intracellular killing • Transfer factor therapy with amphotericin B C – Cardiac abnormalities A - Abnormal face T – T cell defect. Thymus abnormality C – Cleft palate H – Hypocalcemia 22 – Chr 22 Purines Hydroxanthine Uric acid
  • 13. MICROBIOLOGY CLASS Dr.Tarunikaa G PAGE 10 Combined immunodeficiency Wiskott Aldrich syndrome • Defective CI • Abnormal immunoglobulin • Bone marrow transplant • transfer factor • Thymus transplantation AR • Cerebellar ataxia • Telengectasia • Ovarian dysgenesis • Chr abnormality Ataxia telengectasia Nezelof's disease AR ECZEMA T P INFECTION
  • 14. Reticular dysgenesis of De Vaal MICROBIOLOGY CLASS Dr.Tarunikaa G PAGE 11 Precursors of immunocompetent cells in fetal liver and bone marrow Multipotent hematopoietic stem cell SCID Swiss type agammaglobulinemial Lymphoid stem cell
  • 15. SLE C3- Recurrent pyogenic infection C6 C7 C8- Neisserial infection. 01 Component deficiency Disorders of complement C1 inhibitor - Angioneurotic edema C3b inactivator - Pyogenic infeection. 02 Inhibitor deficiency
  • 16. • Enzyme deficiency • Deficiency of opsonic antibody • Drugs • Antineutrohil autoantibodies Disorders of phagocytosis Shwachman disease Actin binding protein deficiency syndrome Hyper IgE syndrome Lazy leucocyte syndrome Tuftsin deficiency Job syndrome Myeloperoxidase deficiency Leucocyte G6PD deficiency
  • 17. CHronic granulomatous infections • Catalase positive pyogenic pathogens - staphylococcus and coliforms • Diminished H2O2 • Defective release of myeloperoxidase • NBT test
  • 18. CHEDIAK HIGASHI SYNDROME • Decreased pigmentation • Photophobia • Nystagmus • Peroxidase positive inclusion bodies
  • 19. MICROBIOLOGY CLASS Dr.Tarunikaa G PAGE 16 Secondary immunodeficiency
  • 20. Can live vaccines be given in immune deficiency patients?
  • 21. Enzyme that is deficient in SCID?
  • 22. What are the 5 B's in X linked agammaglobulinemia?

Editor's Notes

  1. 23.01.2023
  2. 23.01.2023
  3. 23.01.2023